nutrition issues in gastroenterology

Diagnosis, Treatment and NutritionalManagement of Chronic IntestinalPseudo-Obstruction

INTRODUCTION

Chronic intestinal pseudo-obstruction (CIP) is arare and potentially life-threatening disorder ofthe gastrointestinal tract characterized by symp-toms and signs suggestive of mechanical obstructionbut in the absence of a true anatomical lesion. Normal

antegrade propulsive activity of the gastrointestinaltract is defective in CIP; when significant, chronicintestinal failure ensues with an inability to maintainnormal weight and achieve adequate nutrition. Thisdisease entity typically goes unrecognized for longperiods of time before the correct diagnosis is estab-lished. In the interim, patients often undergo repeatedand potentially dangerous tests and treatments. Thismonograph will focus on the following aspects of CIP:understanding the impact of intestinal pseudo-obstruc-tion; describing the etiology and pathophysiology ofCIP; reviewing common symptoms and signs; dis-cussing the accurate diagnosis of CIP; and finally,reviewing treatment options.

NUTRITION ISSUES IN GASTROENTEROLOGY, SERIES #77

Brian E. Lacy, Ph.D., M.D. FACG, Associate Profes-sor of Medicine, Dartmouth Medical School, Director,GI Motility Laboratory, Dartmouth-Hitchcock MedicalCenter, Lebanon, NH. Burr J. Loew, M.D., Fellow inGastroenterology, Division of Gastroenterology andHepatology, Dartmouth-Hitchcock Medical Center,Lebanon, NH.

Chronic intestinal pseudo-obstruction (CIP) is a rare, chronic disorder of the luminal gas-trointestinal tract. Symptoms and signs suggest a mechanical bowel obstruction, althoughin the evaluation of patients with CIP, both routine and specialized tests fail to identifyevidence of mechanical obstruction. Common symptoms include nausea, vomiting, bloat-ing, abdominal distension, and involuntary weight loss. Unfortunately, these symptomsare non-specific, frequently leading to either misdiagnosis or a delay in diagnosis. Manypatients require parenteral nutrition and a large number of patients require chronic opi-oids. This review will focus on the etiology, pathogenesis, diagnosis and treatment ofpatients with CIP.

Carol Rees Parrish, R.D., M.S., Series Editor

9PRACTICAL GASTROENTEROLOGY AUGUST 2009

Brian E. Lacy Burr J. Loew

PRACTICAL GASTROENTEROLOGY AUGUST 200910


Diagnosis, Treatment and Nutritional Management

CASE 1DB is a 43-year-old woman referred for further evalu-ation of abdominal pain, nausea, vomiting, and weightloss. Her past medical history was notable for anepisode of volvulus 10 years earlier that required righthemi-colectomy. After surgery, she had alternatingsymptoms of constipation and diarrhea and waslabeled with the diagnosis of irritable bowel syndrome(IBS). Two years ago she had a viral illness with symp-toms of nausea, vomiting, diarrhea, fever, myalgiasand arthralgias. All of the symptoms except for hernausea and vomiting resolved. Her weight droppedfrom 100 to 70 lbs. She noticed difficulty swallowingliquids and solids. She denied symptoms of anorexiaand bulimia, believed that her weight was too low, anddid not exercise. Physical examination revealed acachectic woman with a BMI of 13.3. Her abdomenwas moderately distended and tympanitic. Blood testswere notable for an albumin of 2.2; her electrolyteswere normal as was a TSH. Her hemoglobin was 10with a normal MCV. She was evaluated by multiplephysicians and underwent a variety of diagnostic tests:

Two separate upper endoscopies were normal Two separate colonoscopies revealed a patent anas-

tomosis without evidence of obstruction An abdominal flat plate while acutely ill showed

dilated loops of small intestine with multiple airfluid levels

A follow-up abdominal x-ray two weeks laterappeared normal

A right upper quadrant ultrasound showed evidenceof prior cholecystectomy but was otherwise normal

Two separate computed tomography (CT) scans ofthe abdomen and pelvis were normal other thandemonstrating post-surgical changes

A small bowel follow-through did not show evi-dence of obstruction however transit was delayed atfour hours

Extensive blood tests, looking for evidence of a con-nective tissue disorder or autoimmune disorder, wereall normal

An MRI of the brain was normal as well Esophageal manometry revealed normal lower

esophageal sphincter (LES) resting pressure of 17mm Hg and complete LES relaxation, but failed peri-

stalsis on nine of 10 water swallows. One swallowwas peristaltic in nature and of normal amplitude

The four hour solid phase gastric emptying scanrevealed 27% of material remaining at four hours(

treated with an over-the-counter PPI with some reliefof heartburn symptoms but no relief of dysphagia. Hisinternist became concerned when he returned for a fol-low-up visit and his weight was 117 lbs. Diagnosticworkup revealed the following:

Laboratory tests were performed (CBC, ESR, elec-trolytes, BUN/Cr, glucose, LFTs, serum TTG anti-body, serum IgA) and all returned normal

Colonoscopy, including random biopsies, was normal Upper endoscopy, including random biopsies of the

duodenum and stomach, were normal A small bowel follow through revealed slow transit

(five hours) through a moderately dilated smallintestine. There was no evidence of obstruction

A laboratory panel for autoimmune disorders or con-nective tissue disorders was obtained (ANA, AMA,anti-smooth muscle antibody, CRP, TSH, SCL-70panel, anti-double stranded DNA, anti-Hu antibody,SPEP). ANA was elevated at a titer of 1:1500, butlabs were otherwise normal

Esophageal manometry revealed a hypotensive LESwith resting pressure of 2 mm Hg, normal UES rest-ing pressure and relaxation, but absent peristalsis inthe body of the esophagus

A four-hour solid phase gastric emptying scan wasdelayed with 37% of the material remaining at fourhours (normal


Schwankovsky and co-workers published qualityof life measurements after a retrospective review of themedical records of 58 patients with congenital CIP (3).Their results demonstrate that a large number of CIPpatients (73%) require central venous access or a per-cutaneous gastrostomy tube in order to maintain ade-quate nutrition. Furthermore, children with CIP,compared to healthy children, had lower levels of self-care and mobility, more difficulty attending school andparticipating in social activities, and more pain, anxi-ety and depression. Parents of children with CIP hadan emotional status rated as poor when compared toparents of healthy children.

The quality of life for adults with intestinal pseudo-obstruction has not been well studied in a prospectivemanner, although it is undoubtedly worse than the gen-eral population and patients with many other chronicdisorders. In one published report, Mann and col-leagues described CIP patients as frequently beingdependent upon supplemental intravenous or enteralnutrition (EN), using multiple expensive medications(often without success), and often becoming dependenton narcotics due to chronic abdominal pain furtheraggravating the pseudo-obstruction symptoms (4).

IDENTIFYING THE CAUSE AND MECHANISM OF DISEASEChronic intestinal pseudo-obstruction is generallygrouped into three categories: primary (either neuro-pathic or myopathic in nature); secondary (due to sys-temic disorders including collagen vascular diseases,endocrine disorders, malignancies, neurologic disor-ders, etc.); or idiopathic (cause unknown). Table 1depicts this classification system and also lists condi-tions commonly associated with CIP.

Normal gastrointestinal motor function is a com-plex sequence of events with an interplay betweennumerous physiological inputs that remain poorlyunderstood. Factors critical to normal gastrointestinaltract function include: intact extrinsic innervation fromthe brain and spinal cord; an intact enteric nervous sys-tem; the presence of normally functioning smooth mus-cle; and normal levels of appropriate gastrointestinalhormones and neurotransmitters. Disruption at anypoint along this complex physiological network may

lead to signs and symptoms suggestive of CIP. It is thuseasy to understand how CIP encompasses such a widespectrum of distinct and variable disease processes withdiffering pathophysiological mechanisms.

The unifying characteristic of CIP is disordered gas-trointestinal tract motility. In primary CIP, whichencompasses the majority of cases, this may stem froman inherent defect in the normal mechanisms that con-trol gastrointestinal tract motility, for example, eitherinjury to the smooth muscle (a myopathic process) or tothe nervous system (a neuropathic process). Damage tothe nervous system in patients with CIP typicallyinvolves injury to the enteric nervous system, althoughinjury may also occur to the autonomic nervous system(either the sympathetic or parasympathetic nerves). Inaddition, within each major group (neuropathic or myo-pathic) CIP can also be categorized into one of threesubcategories: congenital, familial (presumably geneticin nature), or sporadic. Familial visceral myopathy canbe further grouped into type 1 (autosomal dominant),type 2 (autosomal recessive with associated ptosis andophthalmoplegia), or type 3 (autosomal recessive withthe presence of gastrointestinal tract dilation). Thesesubcategories may then be further classified to representareas of intestinal involvement (i.e., colon, small intes-tine, stomach, esophagus or a combination of all four).

A thorough investigation is needed in patientsbeing evaluated for CIP, as a secondary cause that maybe amenable to directed therapies can be identified insome cases. Typical etiologies in this subgroup includecollagen vascular disorders, endocrine disorders, neu-rologic disorders, and medications (Table 1). One ofthe more common collagen vascular diseases to beassociated with CIP is primary systemic sclerosis,which may precede the diagnosis of CIP by severalyears. Other secondary causes of intestinal pseudo-obstruction include amyloidosis and small cell carci-noma of the lung presenting as a paraneoplasticphenomena (5). Viruses have also been implicated as apossible causative factor in CIP (6).

CLINICAL PRESENTATIONAn analysis by Mann and colleagues found that themedian age of symptom onset was 17 years with a rangeof two weeks to 59 years (4). The frequency and sever-



PRACTICAL GASTROENTEROLOGY AUGUST 2009 15



ity of symptoms varies remarkably from patient topatient depending upon the location and the extent ofthe gastrointestinal tract involved (Table 2). The mostcommon symptoms include pain (80%), nausea andvomiting (75%), constipation (40%), and diarrhea(intestinal stasis which promotes bacterial overgrowth;20%) (7). The clinical picture tends to be dominated byabdominal pain and distension which are particularlysevere during episodes of pseudo-obstruction. While thepain may be intermittent and occur only during an acuteepisode or crisis, typically it is chronic in nature. Thepain can be located anywhere in the abdomen, depend-

ing upon the extent and location of the involvedsegment of gastrointestinal tract. Pain typicallyworsens as bloating and abdominal distension pro-gresses and improves as the crisis resolves.

When the esophagus is involved, decreasedesophageal motility and reduced lower esophagealsphincter (LES) tone may lead to complaints ofdysphagia, chest pain and reflux symptoms.

Patients with CIP may also develop problemsoutside of the intestinal tract. The most commonextraintestinal manifestation is genitourinaryinvolvement (7), which presents as dilation of theureter or abnormal bladder function, and commonlyleads to complaints of difficulty urinating (8).

Natural HistoryTwo studies have investigated congenital CIP inchildren presenting within the first year of life andhave demonstrated that 60%80% require par-enteral nutrition (PN) and 10%25% die beforeadulthood (9,10). Most of the adult data is limitedto case reports or small case series so the naturalhistory remains largely unknown. A recentprospective study by Stanghellini and colleaguesfollowed 59 consecutive CIP patients for a medianof 4.6 years (11). The diagnosis of CIP was madea median of eight years after symptoms first devel-oped. During this time each patient underwent anaverage of three surgeries related to their CIP diag-nosis. The long interval of misdiagnosis and themultiple, ineffective and potentially dangeroussurgeries likely occurs for a variety of reasons,including: the rarity of the disease; a general lack

of understanding of the disease; and difficulty in arriv-ing at the diagnosis because symptoms are non-specificand overlap with other more prevalent functional boweldisorders (i.e., functional dyspepsia, idiopathic gastro-paresis, IBS, chronic constipation). Long-term out-comes are poor despite medical and surgical therapies.In the study of 59 patients with CIP described above,four patients died of disease-related complications and4 underwent small bowel transplantation (11). One-third of patients required long-term PN and two-thirdshad nutritional deficiencies or limitations.

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Table 1Classification of Chronic Intestinal Pseudo-Obstruction

Primary Idiopathic **I. Myopathic

A. CongenitalB. FamilialC. Sporadic

II. NeuropathicA. CongenitalB. FamilialC. Sporadic

*Miscellaneous processes may include: celiac disease, infiltrative diseases (amyloid, lymphoma), neoplastic, familial dysautonomia, metabolic (hypokalemia,hypomagnesemia, hypophosphatemia), jejunoileal bypass, mesenteric vascularinsufficiency, alcoholism, viral infections, radiation, post-organ transplant.**Unknown etiology without known precipitant and unrevealing biopsies for a primary mechanism

SecondaryCollagen Vascular Disease Primary Systemic Sclerosis Systemic Lupus Erythematosus Dermatomyositis/Polymyositis Periarteritis nodosa Mixed connective tissue disorder Rheumatoid Arthritis

Endocrine Disorders Hypothyroidism/hypoparathyroidism Diabetes mellitus

Neurological Disorders Parkinsons Disease Hirschsprungs Disease Chagas Disease Intestinal Hypoganglionosis

Drug-Associated Tricyclic antidepressants Anticholinergic agents Ganglionic blockers Anti-parkinsonian agents Phenothiazines Clonidine

Miscellaneous*

Overall, only 11% of CIP patients are asympto-matic between subacute obstructive episodes and donot require chronic medical treatment. Approximately20% of patients develop intractable abdominal painand become opioid dependent (4).

MAKING THE DIAGNOSISUnfortunately, there is no specific biologic marker forCIP, therefore, a complete and thorough history andphysical examination remains the cornerstone of mak-ing an accurate diagnosis. To diagnose CIP, patientsshould have symptoms for a minimum of six months(Table 2). A step-wise approach is used to make thediagnosis of CIP and generally includes pertinent lab-oratory studies, plain films of the abdomen, gastroin-testinal transit measurements, and specialized tests ofgastrointestinal motility (Figure 1).

To begin, patients are evaluated with a battery oflaboratory tests including a complete blood count, ery-throcyte sedimentation rate or C-reactive protein,serum electrolytes including calcium, magnesium andphosphorous, albumin, thyroid stimulating hormone,clotting time (which can be abnormal in patients whohave bacterial overgrowth or severe nutritional prob-lems), and specialized tests (i.e. ANA, AMA, anti-smooth muscle antibody, SPEP, anti-double strandedDNA, SCL-70 panel, anti-Hu) to eliminate the possi-bility of systemic diseases including autoimmuneprocesses, malignancies, and endocrine disorders.

Next, patients should have an abdominal flat plate(KUB) to identify a possible site of obstruction. Thediagnosis of CIP cannot be accurately made if there isno evidence of an obstruction on plain films. Plainfilms obtained during an acute attack typically revealfindings consistent with mechanical obstruction: dis-tended bowel loops and air-fluid levels in the uprightposition. Computed tomography (CT) of the abdomenand pelvis is frequently performed due to symptoms ofpain and concerns over possible mechanical obstruc-tion. The CT scan may be able to identify bowel wallthickening or evidence of blockage or perforation.Barium studies to examine the upper gastrointestinaltract, followed by a barium enema (to assess theanatomy of the colon), are often required to rule outmechanical obstruction and provide evidence of




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Table 2Symptoms and Signs of CIP

Common signs/symptoms Esophageal involvement Abdominal pain Dysphagia Abdominal distension Esophageal reflux/heartburn Bloating Atypical chest pain Nausea/vomiting Constipation Stomach Diarrhea Early satiety

Often due to bacterial overgrowth Extra-intestinal

Weight loss Dysuria Anorexia Abnormal bladder function Nutritional deficiencies Dilated ureter

Figure 1. Algorithm: Diagnosis of CIP. CIP = Chronic intestinalpseudo-obstruction; UGI = Upper gastrointestinal series; SBFT =Small bowel follow-through; BE = Barium enema


intestinal dilation secondary to pseudo-obstruction.Barium studies may also demonstrate a lack of peri-stalsis (seen in myopathic processes) or chaotic peri-stalsis (frequently seen in neuropathic processes).Alternatives to barium studies include water-solublecontrast or using small amounts of barium with aircontrast. Endoscopic evaluation (upper endoscopy,colonoscopy, and capsule endoscopy) can detectmasses, strictures, or physical obstruction, which intheir absence, will help establish the diagnosis of CIP.

Next, tests to measure transit in the gastrointestinaltract are typically obtained. This is most commonly per-formed using a solid phase gastric emptying scan and aSitz mark study (to measure colonic transit). Some spe-cialized motility centers also perform transit studies ofthe small intestine using radioactive materials. Furthersupport for the diagnosis of CIP, and clues to the possi-ble underlying etiology, can often be obtained fromintestinal manometry. Esophageal manometry willreveal abnormalities in esophageal motility in approxi-mately 80% of patients with pseudo-obstruction. Stud-ies with antroduodenal manometry (small bowelmotility) may also reveal characteristic motility abnor-malities. Esophageal manometry is performed at mosthospitals, although antroduodenal manometry is typi-cally performed only at specialized motility centers.

If the physician remains suspicious about the pos-sibility of a mechanical obstruction, then exploratorylaparotomy should be performed. At the same time,full thickness biopsies of the intestinal wall should alsobe obtained. These biopsies will show smooth muscleatrophy in the primary myopathic processes, neuro-pathic degeneration in the primary neuropathic disor-ders, and various findings for the secondary causes ofCIP including fibrosis in primary systemic sclerosis, orevidence of amyloid or lymphoma (Table 3).

TREATMENT OPTIONSChronic intestinal pseudo-obstruction remains a chal-lenge to treat. Therapy for secondary causes of CIP(i.e., scleroderma) should focus on treating the under-lying disorder. This often includes correcting elec-trolyte abnormalities, managing dehydration, treatinginfections, using immunosuppressant agents forpatients with collagen vascular diseases, initiating a

gluten-free diet for pseudo-obstruction associated withceliac disease, or treating the underlying cancer thathas caused a paraneoplastic syndrome.

Treatment for idiopathic or primary CIP, however, isoften quite difficult (Table 4). The disease entity is rareand often goes unrecognized for long periods duringwhich time patients are subjected to numerous treat-ments including repeated surgeries which have thepotential for morbidity and mortality. In contrast to othergastrointestinal disorders, such as acid reflux disease orirritable bowel syndrome, CIP is extremely rare andtherefore large, randomized controlled trials evaluatingdifferent treatment options are lacking, and treatment isbased on clinical experience and the results of smallstudies or individual case reports. In a small, prospectivestudy, only 1 of 20 patients with idiopathic CIP hadsymptomatic improvement with medication therapy (4).

DietIn general, treatment should begin by correcting anynutritional deficiencies that may be present. In order tomaximize oral intake, patients should be encouraged totake in small, frequent meals (five-six per day), with anemphasis on liquids and soft foods, while avoiding highfat solid foods and fiber. Foods high in fat content (>30%total calories) are thought to delay gastric emptying andcause postprandial fullness and nausea, while high fiber



Table 3Histological differences between myopathic and neuropathic CIP*

Myopathic Muscle degeneration Atrophy and fibrosis of one or both layers of the muscularis

propria inflammatory cells

Neuropathic Dense infiltrate of lymphocytes and plasma cells surrounding

myenteric plexus and axons Fragmentation and loss of axons Proliferation of glial cells inflammatory cells

*Some cases may have mixed myopathic and neuropathic histologicalfindings

products are associated with abdominal bloating, bezoarformation, and abdominal discomfort. Lactose oftenneeds to be avoided because of the high incidence of lac-tose intolerance in the general population (25%) and thepotential to worsen abdominal bloating and discomfort.Poorly absorbed sugar alcohols may also aggravatesymptoms (12). Numerous nutritional supplements arecurrently available and are especially useful in malnour-ished patients. A daily multivitamin should be taken, andpatients should receive supplemental essential vitamins,minerals, and electrolytes as needed. Of note, bacterialovergrowth and chronic diarrhea may lead to malabsorp-tion of fat soluble vitamins (A, D, E, and K) and B12deficiency. Referral to a registered dietitian can be veryhelpful for many patients for nutritional education andthe development of a patient specific diet.

Nutrition SupportEnteral Feeding. If dietary changes are unsuccessfulresulting in unmet nutritional requirements and contin-ued weight loss, then enteral nutrition support is the nextstep. In a retrospective study, Scolapio and colleaguesdemonstrated that patients with CIP can generally bemanaged with EN using a standard formula (13). A trialof nasogastric or nasojejunal feeding should be triedprior to placement of a more permanent percutaneousfeeding tube. If patients are able to tolerate fiber-free ENwith few symptoms and regular bowel movements (it isimportant for the clinician to find out the normal stoolhabits of the individual patient), then consideration canbe given for placement of a percutaneous gastrostomyor gastro-jejunostomy tube, or direct placement of ajejunostomy tube, to bypass a dysfunctional stomach. Ifdelayed gastric emptying is present, then direct feedingof the small intestine is preferred, unless the patientwould benefit from having a gastric venting port. Con-tinuous or cycled EN (1012 hours overnight) is oftenbetter tolerated than bolus feedings. A trial of elementalfeeding prior to PN is warranted.

Parenteral Feeding. Ideally, PN should be avoideddue to the risks of cellulitis, sepsis, thrombus forma-tion, and catheter migration or displacement. However,a large proportion of CIP patients will eventuallyrequire parenteral nourishment.




Table 4Treatment of Idiopathic or Primary CIP

Erythromycin, a macrolide antibiotic that acts as an agonist tothe motilin receptor, can be given either orally or intravenously.Doses in the range of 50200 mg orally, or 50100 mg intra-venously (iv), approximately 30 minutes before meals, havebeen shown to be effective in accelerating gastric emptying andimproving symptoms of CIP (16).

Cisapride, a mixed 5HT-4 receptor agonist/5HT-3 receptorantagonist, is not available for routine clinical use, although itcan be obtained in very limited circumstances. Cisapride wasremoved from the market in July 2000 because of drug interac-tions leading to an increased risk of cardiac arrhythmias.

Metoclopramide, a commonly used anti-emetic, is a dopamineantagonist that exerts its prokinetic effects by increasing acetyl-choline release. Metoclopramide is commonly given as 1020mg orally or IV 30 minutes before meals and at bedtime. Mildadverse reactions include fatigue, somnolence, anxiety, jitteri-ness, or depression may occur. More severe adverse eventsincluding extrapyramidal side effects (i.e., tardive dyskinesia) arefortunately, uncommon.

Domperidone is similar to metoclopramide in that it acts as anantagonist at dopamine receptors. Domperidone does not read-ily cross the blood-brain-barrier, and therefore, does not havethe potential for the central nervous system side affects thatmetoclopramide have. Doses range between 1020 mg orally 30minutes before meals and at bedtime. Domperidone is not FDAapproved for use in the United States.

Octreotide, a long acting somatostatin analogue, stimulatessmall intestine motility when given in low doses. It is most effec-tive in patients who have a neuropathic process as the underly-ing etiology of their CIP, since it requires the presence of smoothmuscle in order to be effective. It is usually given in doses of2550 g subcutaneously after both the morning and eveningmeals.

Tegaserod, a specific 5-HT4 receptor agonist, improves gastricemptying, colonic transit, and orocecal transit time, and wasapproved for use only in women with irritable bowel syndromeand constipation (17). Unfortunately, tegaserod is now availableonly for life-threatening emergencies due to concerns that it mayincrease the risk of cardiovascular complications (18).

Lubiprostone, a chloride channel activator approved for thetreatment of chronic constipation and women with IBS and con-stipation, has not been studied in CIP patients (19).


Decompression MeasuresDecompression of distended intestinal segments viaintermittent nasogastric suction, rectal tubes, orendoscopy is helpful for many patients. Given a lackof clinical studies addressing this issue, there are nofirm guidelines on when such intervention should beundertaken. Decompression of the distended intestinalsegment may include a venting enterostomy. Theseare typically placed in the stomach, although somepatients with feeding J-tubes also use them for venting

purposes. As described by Pitt and colleagues, patientswith surgically placed gastrostomy tubes had a lowerrate of hospital admissions (0.2 vs 1.2 admissions perpatient-year) (14), and in another study, admissionsdecreased by 0.5 to 1.0 (15).

Prokinetic AgentsRegardless of whether the underlying process is myo-pathic or neuropathic in nature, all patients with CIPhave disordered gastrointestinal tract motility. Multi-ple prokinetic agents have been used in an attempt topromote normal intestinal motility; however, there arefew investigational studies available to demonstratethe efficacy of any of these agents in CIP (Table 4).

AntibioticsIntestinal stasis may lead to small intestine bacterialovergrowth and diarrhea, with resultant malabsorption,weight loss and the development of multiple vitamindeficiencies. Rotating antibiotics may relieve symp-toms of diarrhea and bloating and improve the nutri-tional status in many patients with CIP. No controlledtrials have been performed to determine which antibi-otics are best, however many clinicians have patientsuse a different antibiotic every month for seven-to-10days over a five-to-six month cycle. (Please see thefollowing review for more information about smallbowel bacterial overgrowth: DiBaise JK. Small Intesti-nal Bacterial Overgrowth: Nutritional Consequencesand Patients at Risk. Practical Gastroenterology,2008;32(12):15).

AntiemeticsPatients with CIP may suffer from recurrent bouts ofnausea and vomiting during an episode of pseudo-obstruction, or they may have nausea on a near dailybasis. There is no single agent particularly suited forthe treatment of nausea and vomiting in CIP. Rather,each patient needs to be assessed individually to deter-mine current medication use, previous trials ofantiemetics, adverse reactions, and financial status.Classes of medications commonly used to treat nauseaare shown in Table 5.



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Table 5Medications Commonly Used to Treat Nausea in CIP

Antihistamines Dimenhydrinate (Dramamine) Promethazine (Phenergan) Meclizine (Antivert) Cyclizine (Marezine) Diphenhydramine (Benadryl)

Anticholinergics Scopolamine

Phenothiazines Prochlorperazine (Compazine) Chlorpromazine (Thorazine) Promethazine (Phenergan)

Butyrophenones Haloperidol (Haldol) Droperidol (Inapsine)

Dopaminergic Antagonists Metoclopramide (Reglan) Domperidone (Motilium)

Serotonin Receptor Antagonists Ondansetron (Zofran) Granisetron (Kytril) Dolasetron (Anzemet)

Miscellaneous Lorazepam (Ativan) Prednisone Dexamethasone Dronabinol (Marinol) Trimethobenzamide (Tigan) Ginger


Small Bowel TransplantationFor patients with intestinal failure and life-threateningPN-related complications, small bowel transplantationhas become an accepted therapy, although data remainslimited. Of four patients who underwent small boweltransplantation for primary CIP, three were alive at 24months and two had improvement in digestive symp-toms after resuming an oral diet, however, all remainedon at least partial parenteral supplementation (11).Intestinal transplantation is only available at a smallnumber of specialized centers in the United States.

CONCLUSIONCIP is a rare, disabling, and potentially life-threateningneuromuscular disorder of the digestive tract simulat-ing mechanical obstruction in the absence of ananatomical lesion. As demonstrated by the two casesprovided above, it often goes unrecognized and misdi-agnosed for long periods of time given its nonspecificsymptoms, clinical overlap with more common condi-tions, and the absence of a biological marker of dis-ease. Maintaining a high index of suspicion togetherwith a careful history and physical examination canguide appropriate further diagnostic testing to arrive atthe correct diagnosis. Unfortunately, except for casesin which a secondary cause can be identified, manage-ment is largely supportive. Ensuring appropriate nutri-tion and managing complications from CIP or itsassociated treatments is paramount. n

References1. Dudley HAF, Sinclair ISR, McLaren IF, et al. Intestinal pseudo-

obstruction. J R Coll Surg Edin, 1958;3:206-217.2. Di Lorenzo C. Pseudo-obstruction: Current Approaches. Gas-

troenterology, 1999;116: 980-987.3. Schwankovsky L, Mousa H, Rowhani A, et al. Quality of life out-

comes in congenital chronic intestinal pseudo-obstruction. DigDis Sci, 2002;47:1965-1968.

4. Mann SD, Debinski HS, Kamm MA. Clinical characteristics ofchronic idiopathic intestinal pseudo-obstruction in adults. Gut,1997;41:675-681.

5. Krishnamurthy S, Schuffler MD. Pathology of neuromusculardisorders of the small intestine and colon. Gastroenterology,1987;93:610-639.

6. Debinski HS, Kamm MA, Talbot IC, et al. DNA viruses in thepathogenesis of sporadic chronic idiopathic intestinal pseudo-obstruction. Gut, 1997;41:100-106.

7. Stanghellini V, Camilleri M, Malagelada JR. Chronic idiopathicintestinal pseudo-obstruction: clinical and intestinal manometricfindings. Gut, 1987;28:5-12.

8. Sullivan MA, Snape WJ, Matarazzo SA, et al. Gastrointestinalmyoelectrical activity in idiopathic intestinal pseudo-obstruction.NEJM, 1977;297:233-238.

9. Mousa H, Hyman PE, Cocjin J, et al. Long-term Outcome ofCongenital Chronic Intestinal Pseudo-obstruction. Dig Dis Sci,2001;47:2298-2305.

10. Faure C, Goulet O, Ategbo S, et al. Chronic Intestinal Pseudo-obstruction syndrome: Clinical Analysis, Outcome and Prognosisin 105 Children. Dig Dis Sci, 1999;44:953-959.

11. Stanghellini V, Cogliandro R, Giorgio R, et al. Natural History ofChronic Idiopathic Intestinal Pseudo-Obstruction in Adults: ASingle Center Study. Clin Gastro Hep, 2005; 3:449-458.

12. Gibson PR, Newnham E, Barrett JS, et al. Review article: fruc-tose malabsorption and the bigger picture. Ali Pharmacol Ther,2007;25:349-363.

13. Scolapio JS, Camilleri M, Romano M. Audit of the treatment ofmalnutrition due to chronic intestinal pseudo-obstruction withenteral nutrition. Nutr Clin Prac, 1999;14:29-32.

14. Pitt HA, Mann LL, Berquist WE, et al. Chronic intestinal pseudo-obstruction: Management with total parenteral nutrition and aventing enterostomy. Arch Surg, 1985; 120:614-618.

15. Murr MM, Sarr MG, Camillieri M. The surgeons role in thetreatment of chronic intestinal pseudoobstruction. Am J Gas-troenterol, 1995;90(12):2147-2151.

16. Minami T, Nishibayashi H, Shinomura Y, et al. Effects of ery-thromycin in chronic idiopathic intestinal pseudo-obstruction.J Gastroenterology, 1996;31:855-859.

17. Lacy BE, Yu S. Tegaserod: A new 5-HT4 agonist. J Clin Gas-troenterology, 2002;34:27-33.

18. http://www.fda.gov/cder/drug/advisory/tegaserod.htm. Accessed2-14-09.

19. Lacy BE, Chey WD. Lubiprostone: chronic constipation and irri-table bowel syndrome with constipation. Expert Opin Pharma-cother, 2009;10:143-152.



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